Periorbital pigmented and reticulated junctional melanocytic naevus: a report of two cases.
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Biomedical subjects
Publications and source records attributed to M N Sotto.
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We demonstrated and quantified by immunohistochemistry the factor XIIIa+ dermal dendrocytes (FXIIIa+ DD) in paracoccidioidomycosis skin lesions. Sixty-one biopsies were classified according to the tissue response in well-organized granulomas (group 1), poorly organized granulomas (group 2) and samples showing both kinds of granuloma (group 3). Ten biopsies from normal skin were used as controls. In order to verify the internalization of Paracoccidioides brasiliensis antigens by FXIIIa+ DD, we performed a double immunostaining technique. FXIIIa+ DD were hypertrophied with prominent dendrites and their number in the test groups was higher than in the control group, especially in the dermal papillae. P. brasiliensis yeasts were seen within the cytoplasm of FXIIIa+ DD in 40% of the immunostained biopsies. We could correlate these findings with the probable role of FXIIIa+ DD as antigen-presenting cells in the pathogenesis of skin lesions in paracoccidioidomycosis.
BACKGROUND: The research on endemic pemphigus foliaceus (fogo selvagem) has mainly focused on the humoral immune response, but little attention has been given to the function of cell-mediated immune response and the nature of the cellular elements of the tissue reaction in the lesions of fogo selvagem. OBJECTIVE: The purpose of this study was the immunophenotype characterization of the inflammatory cells as well as the expression of adhesion molecules and HLA-DR in the perilesional and lesional skin of fogo selvagem. METHODS: Twenty biopsy specimens of lesional and perilesional skin were analyzed by immunohistochemical techniques. The panel of monoclonal antibodies consisted of CD8, CD4, CD1a, HLA-DR, IL-2R, LFA-1, ICAM-1, and PAN-B. RESULTS: The semiquantitative analysis of the cell population revealed a predominance of CD4 T lymphocytes in the tissue response of perilesional and lesional skin. The population of epidermal Langerhans cells was decreased in lesional skin when compared with the perilesional skin, whereas CD1a(+) dermal dendritic cells predominated in lesional skin. Keratinocyte expression of ICAM-1 and HLA-DR was negative in both lesional and perilesional skin. CONCLUSION: The overall results suggest the participation of the cell-mediated immunity in endemic pemphigus foliaceus (fogo selvagem). The lack of keratinocyte ICAM-1 expression may be related to the pattern of cytokines secreted by the CD4(+) T cells of the tissue reaction in fogo selvagem.
Factor XIIIa+ dermal dendrocytes belong to the dermal microvascular unit and are related to wound healing, angiogenic and fibrogenic processes. Erythema elevatum diutinum (EED) is a leukocytoclastic vasculitis followed by repair and fibrosis. In order to verify the involvement of fXIIIa+DD in the pathogenesis of EED and ordinary leukocytoclastic vasculitis (OLV) these cells were immune labeled with anti-factor XIIIa antibody and quantified in 15 biopsies of EED, 18 of OLV and compared with 11 fragments of normal skin (NS). The number of vessels was evaluated by endothelial cell staining with anti CD34 antibody. FXIIIa+DD appeared in both groups of vasculitis with hyperthophic dendrites, with no difference in their number at any level of the dermis. The number of fXIIIa+DD in the superficial dermis was higher in OLV than in NS (p<0.001). The number of dermal vessels in the EED group was higher at all dermis depths evaluated when compared with NS (p<0.05) and in the middle and deep dermis when compared with OLV (p<0.05). The results suggest the participation of fXIIIa+DD in the immunopathological mechanisms of both groups of vasculitis studied. However, there was no correlation between the number of fXIIIa+DD and angiogenesis and fibrogenesis in the EED lesions.
We describe the unique presentation of a linear epidermal nevus with histologic features of Darier's disease occurring in a patient with Gardner's syndrome. Classification of localized forms of Darier's disease as an epidermal nevus or as a genetic mosaicism remains controversial. The association of this disorder with Gardner's syndrome has not been described in the literature before.
Nevus lipomatosus superficialis is a rare disorder characterized by a nevoid fatty growth within the papillary and reticular dermis. Lesions more often occur in the sacral, abdominal, or pelvic regions. A 36-year-old Brazilian female with giant nevus lipomatosus is reported. Our case seems to be the biggest reported in the literature.
Pityrosporum folliculitis is caused by the fungus Pityrosporum ovale. It is characterized by the presence of pruriginous follicular papulae and papulae-pustules in face, upper part of the trunk, and upper limb root. It is more prevailing in places with hot and humid climates. Its incidence can be associated with either immunosuppressive or chemotherapy states secondary to pathologies. We report herewith a case of pityrosporum folliculitis in a patient who had previously underwent kidney transplantation and these result of the itraconazol therapeutics given.
Encephalocraniocutaneous lipomatosis is a rare neurocutaneous syndrome characterized by lipomatous hamartomas ranging in size from a few millimeters to several centimeters and affecting the head. Ocular anomalies and a variable degree of mental retardation with or without convulsions are usually observed. This disorder should be distinguished from other mosaic neurocutaneous phenotypes such as Proteus syndrome, oculocerebrocutaneous syndrome, and nevus sebaceous syndrome. We report the clinicopathologic findings of a 4-year-old Brazilian girl affected by this syndrome and review the literature. To our best knowledge, this is the first documented case of encephalocraniocutaneous lipomatosis occurring sporadically in South America.
BACKGROUND: Patients who have received renal allografts experience early aging of the skin, opportunistic infections, and an increased incidence of skin cancer. OBJECTIVE: We compared the density of lymphocyte subsets and Langerhans cells in normal-appearing skin of renal allograft recipients without skin cancer at 5 to 6 years (group 1) and 14 years after transplant (group 2) with a matched normal control group. METHODS: Biopsy specimens of sun-protected and exposed areas from 18 white, kidney allograft recipients (10 in group 1 and 8 in group 2) with normal renal function and from 10 healthy volunteers were semiquantitatively analyzed for dermal lymphocyte subsets and Langerhans cells. RESULTS: There was a statistically significant decrease in all dermal cell elements in the sun-protected skin of both groups of patients who had received grafts. The sun-exposed skin of group 2 also showed a significant decrease of dermal CD4+ and CD8+ lymphocytes, and group 1 had a significant decrease in dermal CD8+ lymphocytes. The dermal CD1a+ cell population in the sun-exposed skin from both grafted groups did not differ from the control group. CONCLUSION: Kidney transplant recipients showed dermal depletion of cells related to immune surveillance against tumors even before skin cancer occurred, and this depletion seemed to become more marked with the duration of immunosuppression.
Tissue response against inoculation of Leishmania (Leishmania) amazonensis promastigotes in the hind footpad was quite different between two strains of mice: in BALB/c animals there was parasitism of perineurial cells by the 8th week post inoculation (WPI) and heavy parasitism of macrophages, as well as degenerated extracellular parasites close to collagen fibers at the 39th WPI, whereas in C57BI/6j mice there was heavy parasitism of macrophages at 6th WPI, dermal vessels with high endothelial cell at 21st WPI and well preserved intracellular amastigote forms by 51st WPI. In both animals there was no parasitism of keratinocytes or Langerhans cells. Thus BALB/c mice were useful as an experimental model for diffuse cutaneous leishmaniasis and showing a new feature, parasitism of perineurial cells, whereas C57BI/6J animals show hypersensitivity signs, together with a few preserved parasites, only late in the course of infection. From a morphological point of view, there were no differences in macrophages, or in the interaction between this target cell and the parasite, between the animal models studied. This suggests that the difference in the response of the hosts towards the parasite could depend on the way in which they activate a cellular, i.e. lymphocyte mediated immune, response.
BACKGROUND: Pemphigus herpetiformis is a rare and atypical variant of pemphigus that resembles dermatitis herpetiformis. Most patients show antiepidermal autoantibodies that stain the epidermal intercellular spaces by immunofluorescence, similar to pemphigus autoantibodies, and lack the immunopathologic features of dermatitis herpetiformis. OBJECTIVE: The study was aimed at characterizing the specificity of the antiepidermal autoantibodies in seven patients with pemphigus herpetiformis. METHODS: The antiepidermal autoantibodies were characterized by immunofluorescence, immunoblotting, and immunoprecipitation studies in seven patients who fulfilled the clinical, histologic, and immunofluorescence diagnostic criteria for pemphigus herpetiformis. RESULTS: Five patients with features of pemphigus herpetiformis either had classic pemphigus foliaceus, or their disease evolved into classic pemphigus foliaceus. One of these patients had fogo selvagem. Two of the seven patients showed features of or had disease that evolved into pemphigus vulgaris. The antiepidermal autoantibodies present in all seven patients recognized desmoglein 1. CONCLUSION: Pemphigus herpetiformis is a rare clinical and histologic expression of nonendemic pemphigus foliaceus, fogo selvagem, and pemphigus vulgaris.
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Tecidual reaction at the inoculation site of L. (L.)chagasi promastigotes in hamsters depleted and non-depleted of complement was studied within 2, 6, 12, 24, 48 and 72 hours of infection. The inflammatory reaction was characterized by early predominance of polymorphonuclear cells (PMN) at 2, 6 and 12 hours of infection, mixed infiltrate of PMN and mononuclear cells (MN) at 24 hours, followed by predominance of MN at 48 and 72 hours after infection. The group depleted of complement showed a higher number of PMN at 2 hours and lower numbers of MN at 72 hours after infection (P < 0.0001). In the depleted group the phagocytosis by PMN was lower at 2 and 24 hours and by MN was lower at 24, 48 and 72 hours after infection. Electron microscopy showed extracellular intact and degenerated parasites, and lysed intracellular parasites, in PMN; and, rarely, preserved intracellular parasites in MN at 2, 6 and 12 hours after infection. The groups examined at 24, 48 and 72 hours of infection showed only cellular and parasite debris in mononuclear inflammatory cells. C3b deposits were detected by immunofluorescence in the interstitium and in the cytoplasm of inflammatory cells in non-depleted group at 2, 6 and 12 hours of infection. No immunoglobulin was detected in either group. Visceralization was detected 240 days after infection. The complement system has an important role in the inflammatory reaction and phagocytosis. The ultrastructural findings showed that the escape of the parasite probably occurs soon after inoculation.
We describe a woman with sclerotic and atrophic lesions of the breast. Histopathologic and immunologic findings indicated a diagnosis of lupus erythematosus. Lupus mastitis is a rare presentation of panniculitis; only a few cases have been reported in the literature.
Biopsy specimens of cutaneous discoid lesions of 71 patients with cutaneous lupus erythematosus (CLE) were studied. The material was examined by direct immunofluorescence (DIF) to establish positivity and morphologic patterns of immunoglobulins and complement deposits in the basement membrane zone (BMZ). A correlation between DIF results and thickening of the epidermis basement membrane (BM) stained by periodic acid-Schiff (PAS), obtained from 31 matched biopsy specimens, also is presented. Direct immunofluorescence had positive results in 66.20% of the 71 examinations and 70.97% of the matched examinations, whereas PAS showed BM thickening in 100% of the specimens. This observation stresses the importance of such histopathologic findings in the diagnosis of discoid lesions in CLE. There was no correlation between DIF patterns and PAS-stained BMZ thickening. These findings merit additional study. In conclusion, an accordance between PAS-stained BM thickening and immunoglobulin deposits has occurred in 70.97% of cases. This phenomenon does not depend on the presence of immunocomplexes, for it occurs even in cases in which immunocomplex deposits were not detected.
AIMS: To compare the efficacy of histochemical and immunohistochemical methods in detecting forms of Sporothrix schenckii in tissue. METHODS: Thirty five cutaneous biopsy specimens from 27 patients with sporotrichosis were stained by histochemical haematoxylin and eosin, periodic acid Schiff, and Gomori's methenamine silver methods and an immunohistochemical (avidin-biotin complex immunoperoxidase) (ABC) technique associated with a newly produced rabbit polyclonal antibody anti-Sporothrix schenckii. RESULTS: A total of 29 (83%) cases were positive by the ABC method used in association with anti-Sporothrix schenckii rabbit polyclonal antibodies. Histochemical methods, using silver staining, periodic acid Schiff, and conventional haematoxylin and eosin detected 37%, 23%, and 23% of forms of S schenckii, respectively. The ABC technique was significantly more reliable than periodic acid Schiff and silver staining techniques. CONCLUSIONS: It is concluded that immunostaining is an easy and rapid method which can efficiently increase the accuracy of the diagnosis of sporotrichosis in human tissue.
GAPO syndrome was described in 12 patients from 7 families. Constant manifestations include dwarfism, alopecia, pseudoanodontia, and a peculiar, "geriatric" facial appearance. We describe the autopsy findings and all available clinical data on one deceased patient and his living affected sister, previously reported as short abstracts (Epps et al.: Cienc Cult 29(Suppl):740, 1977; Wajntal et al.: Cienc Cult 34(Suppl):705, 1982). Both had the characteristic anomalies of this syndrome but optic atrophy was absent; instead, they had glaucoma and keratoconus; hypogonadism was present in both patients. Biopsy and autopsy findings show that the GAPO syndrome is a dyshistogenetic sequence due to accumulation of extracellular material and thus should be called GAPO dysplasia. We suggest that the basic defect in this autosomal recessive disorder is possibly related to a lack of breakdown of the extracellular components, perhaps due to an enzyme deficiency involved in the metabolism of extracellular matrix.
A case of hyalohyphomycosis caused by Paecilomyces lilacinus is described in a renal transplant patient. Infection was localized on the left forearm. Two separate cultures of the lesions yielded the same organism. Histological sections disclosed hyaline elements in the tissue. The infection responded well to therapy with oral griseofulvin (500 mg daily) and the patient was cured within 45 days. Forty-six cases of human infection due to Paecilomyces species have been reported previously in the literature, most of them occurring in conjunction with prosthesis implants or immunosuppression. The antifungal sensitivity of Paecilomyces varies widely among the species but the general trend is for Paecilomyces variotii isolates to be almost universally sensitive to amphotericin B and 5-fluorocytosine, while P. lilacinus and Paecilomyces marquandii isolates are resistant to these antifungals but sensitive to the imidazoles.